Leprosy

Leprosy proper, or lepra tuberculosa, in contradistinction to other skin diseases commonly designated by the Greek word lepra (psoriasis, etc.), is a chronic infectious disease caused by the bacillus leprœ, characterized by the formation of growths in the skin, mucous membranes, peripheral nerves, bones, and internal viscera, producing various deformities and mutilations of the human body, and usually terminating in death.

History of the disease

Leprosy was not uncommon in India as far back as the fifteenth century B.C. (Ctesias, Pers., xli; Herodian, I, i, 38), and in Japan during the tenth century B.C. Of its origin in these regions little is known, but Egypt has always been regarded as the place whence the disease was carried into the Western world. That it was well known in that country is evidenced by documents of the sixteenth century B.C. (Ebers Papyrus); ancient writers attribute the infection to the waters of the Nile (Lucretius, "De Nat. rer.", VI, 1112) and the unsanitary diet of the people (Galen). Various causes helped to spread the disease beyond Egypt. Foremost among these causes Manetho places the Hebrews, for, according to him, they were a mass of leprosy of which the Egyptians rid their land (" Hist. Græc. Fragm.", ed. Didot, II, pp. 578-81). Though this is romance, there is no doubt but at the Exodus the contamination had affected the Hebrews. From Egypt Phœnician sailors also brought leprosy into Syria and the countries with which they had commercial relations, hence the name "Phœnician disease" given it by Hippocrates (Prorrhetics, II); this seems to be borne out by the fact that we find traces of it along the Ionian coasts about the eighth century B.C. (Hesiod, quoted by Eustathius in "Comment. on Odyss.", p. 1746), and in Persia towards the fifth century B.C. (Herodotus). The dispersion of the Jews after the Restoration (fifth century) and the campaigns of the Roman armies (Pliny, "Hist. Nat.", XXVI) are held responsible for the propagation of the disease in Western Europe: thus were the Roman colonies of Spain, Gaul, and Britain soon infected.

In Christian times the canons of the early councils (Ancyra, 314), the regulations of the popes (e.g., the famous letter of Gregory II to St. Boniface), the laws enacted by the Lombard King Rothar (seventh century), by Pepin and Charlemagne (eighth century), the erection of leper-houses at Verdun, Metz, Maestricht (seventh century), St. Gall (eighth century), and Canterbury (1096) bear witness to the existence of the disease in Western Europe during the Middle Ages. The invasions of the Arabs and, later on, the Crusades greatly aggravated the scourge, which spared no station in life and attacked even royal families. Lepers were then subjected to most stringent regulations. They were excluded from the church by a funeral Mass and a symbolic burial (Martène, "De Rit. ant., " III, x). In every important community asylums, mostly dedicated to St. Lazarus and attended by religious, were erected for the unfortunate victims. Matthew Paris (1197-1259) roughly estimated the number of these leper-houses in Europe at 19,000, France alone having about 2000, and England over a hundred. Such lepers as were not confined within these asylums had to wear a special garb, and carry "a wooden clapper to give warning of their approach. They were forbidden to enter inns, churches, mills, or bakehouses, to touch healthy persons or eat with them, to wash in the streams, or to walk in narrow footpaths" (Creighton). (See below: IV. Leprosy in the Middle Ages.) Owing to strict legislation, leprosy gradually disappeared, so that at the close of the seventeenth century it had become rare except in some few localities. At the same time it began to spread in the colonies of America and the islands of Oceanica. "It is endemic in Northern and Eastern Africa, Madagascar, Arabia, Persia, India, China and Japan, Russia, Norway and Sweden, Italy, Greece, France, Spain, in the islands of the Indian and Pacific Oceans. It is prevalent in central and South America, Mexico, in the West Indies, the Hawaiian and Philippine islands, Australia and New Zealand. It is also found in New Brunswick, Canada. In the United States, the majority of cases occur in Louisiana and California, while from many other States cases are occasionally reported, notably from New York, OhioPennsylvania, Minnesota, Missouri, the Carolinas and Texas. In Louisiana leprosy has been gaining foothold since 1758, when it was introduced by the Acadians" (Dyer). According to the statistics furnished by delegates to the second international conference on leprosy (at Bergen, Norway, Sept., 1909), there are approximately 200,000 cases of the disease throughout the world: India, it is stated, coming first with 97,340 cases; the United States contributing 146 cases, and the Panama Canal Zone the minimum of 7 cases.

Pathology

How leprosy originated is unknown: bad nutrition, bad hygiene, constitutional conditions (tuberculosis, alcoholism, probably heredity, etc.) seem to favour its production and propagation. The disease is immediately caused by the infection of the bacillus leprœ, a small rod bacillus from 003 mm. to .007 mm. in length and .0005 mm. in diameter, straight or slightly curved, with pointed, rounded, or club-shaped extremities, usually found in short chains or beads. This bacillus, discovered in 1868 by Hansen, has been described since 1880 by many specialists, particularly by Byron, who succeeded in cultivating it in agar-agar (Ceylon moss). It is present in all leprous tissues and the secretions (urine excepted; Köbner claims to have seen it in the blood), and has been repeatedly observed in the earth taken from the graves of lepers (Brit. Lepr. Commission of India). There is on record only one case — and this somewhat doubtful — of leprosy communicated by artificial inoculation. As to whether it is contagious from person to person, this was for years a much mooted question among specialists; although a scientific demonstration of contagiousness is so far impossible — the mode of contamination being as yet unascertained, as well as the period of incubation of the germ — still there are unimpeachable practical proofs of contagion, such as the effect of isolation on the spread of the disease, and cases of healthy persons contracting the disease when exposed (Fathers Damien and Boglioli, nurses, and attendants), even accidentally, as in the instance of a medical student who cut himself while making a post-mortem on a leper. In the international conference at Bergen, these evidences were deemed convincing enough to call for a declaration that the disease be considered contagious.

The period of incubation is "estimated at from a few weeks to twenty and even forty years" (Dyer). Like most infections, leprosy has a preliminary stage, uncertain in its character: there are loss of appetite, dyspepsia, and nausea, neuralgia, rheumatic and articular pains, fever, intermittent or irregular, unaccountable lassitude and anxiety. These premonitory symptoms, which may last for months, are followed by periodical eruptions. Blotches, first reddish, then brown with a white border, appear and disappear in various parts of the body; sooner or later small tumours, filled with a yellowish substance fast turning to a darker hue, rise sometimes on the joints, but oftener on the articulations of the fingers and toes. These tumours, however, are not yet specifically leprous; at the end they may leave permanent spots, pale or brown, or nodules. Then the disease, manifested by the apparition of specifically leprous formations, diverges into different varieties, according as it affects the skin and mucous membranes (cutaneous leprosy), or the nerves (anæsthetic), or both (mixed, or complete); each of these varieties, however, merges frequently into the others, and it is sometimes difficult to draw the line between cases.

Cutaneous leprosy is either macular or tubercular. The former variety is characterized by dark (L. maculosa nigra), or whitish (L. m. alba) spots, usually forming on the place of the old blotches; the eruption, at first only intermittent, turns finally into an obstinate ulcer with constant destruction of tissue; the ulceration usually begins at the joints of the fingers and toes, which drop off joint by joint, leaving a well-healed stump (L. mutilans); it is sometimes preceded by, and ordinarily attended with, anæsthesia, which, starting at the extremities, extends up the limbs, rendering them insensible to heat and cold, pain, and even touch. In the tubercular type, nodosities of leprous tissue, which may reach the size of a walnut, are formed out of the blotches. They may occur on any part of the body, but usually affect the face (forehead, eyelids, nose, lips, chin, cheeks, and ears), thickening all the features and giving them a leonine appearance (leontiasis, satyriasis). Tubercular leprosy develops rapidly, and, when attacking the extremities, its destructive process has the same effect of ulceration, mutilation, and deformity as has been mentioned above. Scarcely different from the preceding in the period of invasion is the course of anæsthetic leprosy, one of the characteristic symptoms of which is the anæsthesia of the little finger, which may occur even before any lesions appear. The ulcer, at first usually localized on one finger, attacks one by one the other fingers, then the other hand; in some cases the feet are affected at the same time, in others their ulceration follows that of the hands. Neuralgic pains accompany the invasion, and a thickening of certain nerves may be observed; motor-paralysis gradually invades the face, the hands, and the feet. Consequent upon this, the muscles of the face become contracted and distorted by atrophy; ectropion of the lower lids prevents the patient from shutting his eyes; the lips become flabby, and the lower one drops. The sense of touch and muscle-control being lost, the hands are unable to grasp, and the contraction affecting the muscles of the forearm produces the claw-hand. In the lower extremities analogous effects are produced, resulting first in a shuffling gait and finally in complete incapacity of motion. Then the skin shrinks, the hair, teeth, and nails fall, and the lopping-off process of necrosis may extend to the loss of the entire hand or foot. The mixed variety of leprosy is the combination and complete development of the two types just described. In all cases a peculiar offensive smell, recalling that of the dissecting-room mixed with the odour of goose feathers — the authors of the Middle Ages compared it to that of the male-goat — is emitted by the Leper, and renders him an object of repulsion to all who come near him. Add the torture of an unquenchable thirst in the last stages of the disease, and, as the patient usually preserves his mind unaffected to the end, the utter prostration resulting from his complete helplessness and the sight of the slow and unrelenting process of decomposition of his body, and it is easy to understand how truly, in the Book of Job (xviii, 13), leprosy is called "the firstborn of death".

The average course of leprosy is about eight years, the mixed type being more rapidly concluded. "Death is the ordinary conclusion of every case, which may come (in 38 per cent of cases) from the exhaustive effects of the disease, from an almost necessary septicæmia, or from some intercurrent disease, as nephritis (in 22.5 per cent); from pulmonary diseases including phthisis (in 17 per cent), diarrhœa (in 10 per cent), anæmia (in 5 per cent), remittent fever (in 5 per cent), peritonitis (in 2.5 per cent)" (Dyer).

So far leprosy has baffled all the efforts of medical science: almost every conceivable method of treatment has been attempted, yet with no appreciable success. Occasionally the treatment has been followed by such long periods of remission of the disease (fifteen or twenty years) as might lead one to believe the cure altogether complete; still, specialists continue to hold that in such instances the virulence of the bacillus is, through causes unknown, merely suspended, and may break forth again. It being admitted that the disease is both contagious and preventible, there seems to be no doubt that means of public protection should be provided. To answer this purpose, several countries (Norway and Sweden in particular) have by legislation ordered the isolation of lepers. In some other countries the Governments encourage, and, more or less generously, subsidize private establishments. Of all the states of the Union, Louisiana is the only one to have taken any definite steps: it partly supports the leper-home at Carville where some seventy patients are housed under the care of the Sisters of Charity of St. Vincent de Paul (Emmitsburg). Some, not unwisely, think that if the federal authorities do not deem it right to interfere, individual states, especially those which, like California are exposed to a constant danger of infection, should take means of preventing the spread of the disease.

[Note: In fact leprosy has been easily and completely curable using multi-drug therapy (MDT) for many years now. Using MDT, the global prevalence of the disease has fallen by 90% since 1985 with over 12 million patients cured worldwide since that date. Moreover, the gross disabilities which were such a feature of the disease for millennia (and are featured so often in the Bible) are now becoming a thing of the past, with early detection and treatment. Leprosaria and the physical separation of patients from their families are completely unnecessary.]

Leprosy in the Bible

The foregoing sketch of the pathology of leprosy may serve to illustrate some of the many passages of the Bible where the disease is mentioned. From the epoch of the sojourn of the people of God in the desert down to the times of Christ, leprosy seems to have been prevalent in Palestine: not only was it in some particular cases (Numbers 12:10; 2 Kings 5:27; Isaiah 53:4) looked upon as a Divine punishment, but at all times the Hebrews believed it to be contagious and hereditary (2 Samuel 3:29); hence it was considered as a cause of defilement, and involved exclusion from the community. From this idea proceeded the minute regulations of Lev., xiii, xiv, concerning the diagnosis of the disease and the restoration to social and religious life of those who were cleansed. All decisions in this matter pertained to the priest, before whom should appear personally both those who were suspected of leprosy and those who claimed to be healed. If, at the first examination, the signs — coloured nodule, blister, shining spot (xiii, 2), discoloration of the hair (3) — were manifest, isolation was pronounced at once; but if some of the signs were wanting, a seven-days quarantine was ordered, at the term of which a new inspection had to take place; should then the symptoms remain doubtful, another week's quarantine was imposed. The appearance of "the living flesh" in connexion with whitish blotches was deemed an evident sign of the infection (10). White formations covering the whole body are no sign of leprosy unless "live flesh" (ulceration) accompany them; in the latter case, the patient was isolated as suspect, and if the sores, which might be only temporary pustules, should heal up, he had to appear again before the priest, who would then declare him clean (12-17). A white or reddish nodule affecting the cicatrix of an ulcer or of a burn would be regarded a doubtful sign of leprosy, and condemned the patient to a seven-days quarantine, after which, according as clearer signs appeared or not, he would be declared clean or unclean (18-28). Another suspicious case, to be re-examined after a week's seclusion, is that of the leprosy of the scalp, in which, not leprosy proper, but ringworm should most likely be recognized. In all cases of acknowledged leprous infection, the patient was to "have his clothes hanging loose, his head bare, his mouth covered with a cloth" and he was commanded to cry out that he was defiled and unclean. As long as the disease lasted, he had to "dwell alone without the camp" (or the city). Like the presence of leprosy, so the recovery was the object of a sentence of the priest, and the reinstatement in the community was solemnly made according to an elaborate ritual given in Lev., xiv.

In connexion with leprosy proper, Leviticus speaks also of the "leprosy of the garments" (xiii, 47-59) and "leprosy of the house" (xiv, 34-53). These kinds of leprosy, probably due to fungus formations, have nothing to do with leprosy proper, which is a specifically human disease.

Leprosy in the Middle Ages

As a consequence of the dissemination of leprosy in Europe, legislation providing against the spread of the disease (which was considered to be contagious) and regulations concerning the marriage of leprous persons, as well as their segregation and detention in institutions — which were more charitable and philanthropic than medical, partaking of the character of asylums or almshouses — gradually came into operation. The historical researches of Virchow concerning leper-houses (leprosoria) have established the fact that such institutions existed in France as early as the seventh century at Verdun, Metz, Maestricht, etc., and that leprosy must even then have been widespread. In the eighth century St. Othmar in Germany and St. Nicholas of Corbis in France founded leper-houses, and many such existed in Italy. (See Virchow in "Archiv für pathologische Anatomie", XVIII-XX, Leipzig, 1860.) Legislative enactments against the marriage of lepers, and providing for their segregation, were made and enforced as early as the seventh century by Rothar, King of the Lombards, and by Pepin (757) and Charlemagne (789) for the Empire of the Franks. The earliest accounts of the founding of leper-houses in Germany is in the eighth and ninth century; in Ireland (Innisfallen), 869; England, 950; Spain, 1007 (Malaga) and 1008 (Valencia); Scotland, 1170 (Aldnestun); the Netherlands, 1147 (Ghent). The founding of these houses did not take place until the disease had spread considerably and had become a menace to the public health. It is said to have been most prevalent about the time of the Crusades, assuming epidemic proportions in some localities: in France alone, at the time of the death of Louis IX, it was computed that there were some two thousand such houses, and in all Christendom not less than nineteen thousand (Hirsch, "Handbook of Geographical and Historical Pathology", tr. Creighton, London, 1885, p. 7, note. Cf. Raymund) "Histoire de l'Eléphantiasis", Lausanne, 1767, p. 106). Mézeray (Hist. de France, II, 168) says: "Il y avait ni ville ni bourgade, que ne fust obligée de bâtir un hôpital pour les (lepreux) retirer". For Italy we have Muratori's statement (Antiq. Ital. Med. Ævi, III, 53), "Vix ulla civitas quæ non aliquem locum leprosis destinatum haberet."

There is, however, good reason to doubt the accuracy of the above figures (19,000) as estimated by our medieval informants. Besides, "it would be a mistake", writes Hirsch (op. cit., p. 7), "to infer from the multiplication of leper-houses, that there was a corresponding increase in the number of cases, or to take the number of the former as the measure of the extent to which leprosy was prevalent, or to conclude, as many have done, that the coincidence of the Crusades implies any intrinsic connexion between the two things; or that the rise in the number of cases was due to the importation of leprosy into Europe from the East. In judging of these matters we must not leave out of sight the fact that the notion of 'leprosy' was a very comprehensive one in the middle age, not only among the laity but also among physicians; that syphilis was frequently included therein, as well as a variety of chronic skin diseases, and that the diagnosis with a view to segregating lepers was not made by the practitioners of medicine but mostly by the laity."

Simpson, in his admirable essay on the leper-houses of Britain (Edin. Med. and Surg. Journal, 1841-42), writes: "I have already alluded to special Orders of Knighthood having been established at an early period for the care and superintendence of lepers. We know that the Knights of St. Lazarus separated from the general Order of the Knights Hospitallers about the end of the eleventh or beginning of the twelfth century (Index. Monast., p. 28). They were at first designated: Knights of St. Lazarus and St. Mary of Jerusalem. St. Louis brought twelve of the Knights of St. Lazarus to France and entrusted them with the superintendence of the 'Lazaries' (or leper hospitals) of the Kingdom. The first notice of their having obtained a footing in Great Britain is in the reign of Stephen (1135-54) at Burton Lazars (Leicestershire). I find that the hospitals of Tilton, of the Holy Innocents at Lincoln, of St. Giles (London), Closely in Norfolk, and various others are annexed to Burton Lazars as 'cells' containing 'fratres leprosos de Sancto Lazaro de Jerusalem'. Its [Burton's] privileges and possessions were confirmed by Henry II, King John and Henry VI. It was at last dissolved by Henry VIII." (See ORDER OF ST. LAZARUS.)

As has already been stated, these institutions were intended principally as houses to seclude the infected, and not so much as hospices for the curative treatment of the disease, which was considered then, as now, an incurable disorder. They were founded and endowed as religious establishments, and as such they were generally placed under the control and management of some abbey or monastery by a papal Bull, which appointed every leper-house to be provided with its own churchyard, chapel, and ecclesiastics — "cum cimuterio ecclesiam construere et propriis gaudere presbyteris" (Semler, "Hist. Eccles. Select."). The English and Scotch houses were under the full control of a custos, dean, prior, and, in some cases — as in the hospital of St. Lawrence, Canterbury, which contained lepers of both sexes — a prioress. The ecclesiastical officers of the hospitals and the leper inmates were bound by the regulations laid down in the charters of the institution, which they had to observe strictly, especially as to offering up prayers for the repose of the souls of the founder and his family. The following extracts from the regulations of the leper-hospital at Illeford (Essex), in 1346, by Baldock, Bishop of London, illustrate this point: "We also command that the lepers omit not attendance at their church, to hear divine service unless prevented by previous bodily infirmity, and they are to preserve silence and hear matins and mass throughout if they are able; and whilst there to be intent on devotion and prayer as far as their infirmity permit them. We advise also and command that as it was ordained of old in the said hospital every leprous brother shall every day say for the morning duty, an Our Father and Hail Mary thirteen times and for the other hours of the day . . . respectively an Our Father and a Hail Mary seven times, etc. . . . If a leprous brother secretly [occulte] fails in the performance of these articles let him consult the priest of the said hospital in the tribunal of penance" (Dugdale, "Monasticon Anglicanum", II, 390). There was generally a chaplain under the prior and in some instances a free chapel was attached with resident canons. The hospital at St. Giles (Norwich), for instance, had a prior and eight canons (acting chaplains), two clerks, seven choristers, and two sisters (Monast., Index, 55).

Matthew Paris has left us a copy of the vow taken by the brothers of the leper-hospitals of St. Julian and St. Alban before admission: "I, brother B., promise and, taking my bodily oath by touching the most sacred Gospel, affirm before God and all the Saints in this church which is constructed in honour of St. Julian (the Confessor), in the presence of Dominus R. the archdeacon, that all the days of my life I will be subservient and obedient to the commands of the Lord Abbot of St. Albans for the time being and to his archdeacon, resisting in nothing, unless such things should be commanded as could militate against the Divine pleasure: I will never commit theft, or bring a false accusation against any one of the brethren, nor infringe the vow of chastity nor fail in my duty by appropriating anything, or leaving anything by will to others, unless by a dispensation granted by the brothers. I will make it my study wholly to avoid all kinds of usury as a monstrous thing and hateful to God. I will not be aiding or abetting in word or thought, directly or indirectly in any plan by which any one shall be appointed Custos or Dean of the lepers of St. Julians, except the persons appointed by the Lord Abbot of St. Albans. I will be content, without strife or complaint, with the food and drink and other things given and allowed to me by the Master; according to the usage and custom of the house. I will not transgress the bounds prescribed to me, without the special license of my superiors, and with their consent and will; and if I prove an offender against any article named above, it is my wish that the Lord Abbot or his substitute may punish me according to the nature and amount of the offence, as shall seem best to him, and even to cast me forth an apostate from the congregation of the brethren without hope of remission, except through special grace of the Lord Abbot." It is interesting to compare with the passage on usury in this formula the statement of Mézeray (Hist. de France), that during the twelfth century two very cruel evils (deux maux très cruels) reigned in France, viz., leprosy and usury, one of which, he adds, infected the body while the other ruined families.

The Church, therefore, from a remote period has taken a most active part in promoting the wellbeing and care of the leper, both spiritual and temporal. The Order of St. Lazarus was the outcome of her practical sympathy for the poor sufferers during the long centuries when the pestilence was endemic in Europe. Even in our own day we find the same Apostolic spirit alive. The saintly Father Damien, the martyr of Molokai, whose life-sacrifice for the betterment of the lepers of the Sandwich Islands is still fresh in public recollection, and his co-labourers and followers in that field of missionary work have strikingly manifested in recent times the same apostolic spirit which actuated the followers of St. Lazarus in the twelfth and two succeeding centuries.

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